Doctors call them heart sounds. The sounds they hear as blood flows through the heart, as valves open and close.
There are some other heart sounds, in my own heart. They are the sounds, the lives of those born with bicuspid aortic valves. Others have another kind of aortic disease in their chests. They also have entrusted their life stories to me. Too precious to be lost or ignored, I share their heart sounds with you here.

Sunday, April 9, 2017

It is 27 years this month since my husband's own bicuspid aortic valve was replaced. It had completely failed him, causing his entire heart to fail too.

Since that time, we know now that his body has struggled with the artificial valves that have kept him alive, aortic valves made by man. First a mechanical valve. Then a bovine tissue vale. And now, it is almost 6 weeks since he received his third artificial aortic valve.

Why another surgery? He was, quite simply, on the brink of losing his life. The 11 year old bovine prosthetic valve in his heart was failing.

The 2 leaflets at the bottom were completely frozen,
only the 1 leaflet at the top was moving to let blood flow

Tissue Valve Deteriorating at Eight Years
At 8 years, we were told that one of the leaflets was not moving well. Only 8 years?

We had hoped that he would be like many in his age group, whose bovine pericardial valves still function at 20 years.

Inside his body, for whatever the reason, the valve is considered a foreign invader, something that needs to be attacked or healed!

I had read that tissue valves like this last about 6 years in children, and that was the case for one teenager we know.

He had a transesophageal echo, and those findings was more hopeful. The one leaflet in trouble was moving enough to close completely, it just could not open all the way. Maybe it wasn't too bad, and would still last quite a long time.

Another Leaflet in Trouble

Late in 2016, with the valve approaching 11 years old, a "regular" echocardiogram, through the chest wall, showed that two leaflets were in trouble now. We had to face that it wasn't just one leaflet, and that this valve continued to deteriorate.

Surgery the Only Hope

My husband was not a candidate for TAVI (TAVR), the procedure where another valve is threaded up and inserted inside the old, failing valve. One of the reasons for that was that his aortic valve diameter is too small, only 21 mm, to accept another valve inside it.

There was another reason also, that would have prevented TAVI from helping him. On the bottom, or "intake" side of the valve, my husband had grown scar tissue, called pannus all around the ring of the valve. It is pictured on the left. Most of the tissue was cut away and sent to pathology, so only a small part of the softer tissue remains attached.

This tissue was also blocking blood flow. In order to have full blood flow, those failing valve leaflets, and the scar tissue attached to the valve ring, had to come out. There was no other way.

Today, understanding these things, I am surprised that my husband's heart and body had coped as long as it had.

I found a paper from surgeons in Japan, Subvalvular Pannus Overgrowth after Mosaic Bioprosthesis Implantation in the Aortic Position, that discusses this happening in some of their patients. There is a picture, Figure 1, that shows the ring of scar tissue below the valve. In the conclusion, it mentions that preventing this scar tissue formation is unsolved.My husband also grew pannus and strands of tissue on his first prosthetic valve, a mechanical valve. My personal feeling is that there is something about his body's reaction to these "foreign" valves that causes it to want to heal. However, in the body's attempts to heal, it has hurt him.I do not know how many people form scar tissue on their artificial valves. Perhaps some of them do not live long enough.In any case, this is why time was no longer our friend, and the surgery door was our door of hope. No Solutions?And that, in this year 2017, remains the challenge. There is still far too little that is understood about the tissue in some of those who are apparently most complex, like my husband, who were born with a bicuspid aortic valve. For the sake of generations following us, we need answers to these mysteries.I am glad that physicians such as those in Japan have seen and published about this. I refuse to believe that there are no solutions to these problems, if only someone will look for them!

Monday, April 3, 2017

An Unplanned Door of Hope The emergency entrance of the hospital where my husband had his recent heart surgery.

As we passed the three week milestone after his surgery, there were some signs that my husband was not progressing as he should. Nothing, however, prepared us for his sudden decline. In the early hours of the morning, I was online and on the phone, searching for an ambulance to return him to the hospital where his surgery was performed.

The Nearest Hospital

I could have called 911, the emergency telephone number here in the US. If I had done so, an ambulance and fire truck would have come quickly, from less than a mile away.

Why didn't I just do that - call 911? From previous experience, I knew that our local service would take him to the nearest hospital that in their judgment had the expertise he needed. If you search online, you will find articles that describe how this works, in my area as well as most of the US.

Continuity of Care

Quite simply, I didn't call them because I felt strongly that my husband needed to be back under the care of the same team that had so recently cared for him during and following his heart surgery. They were not the physicians nearest to us, but they were best equipped to understand and help him.

In the medical world, they call this continuity of care.

Finding an Ambulance

Some companies would only transport him to the nearest facility. I found one service that would not do it, because it had to be a distance of at least 300 miles, and we only needed to go about 75!

Who would do it? I found an "inter facility transportation" service. Yes, they would take him from our home to the Emergency Room of the hospital where he had surgery.

I am forever grateful to this company.

Decision in My Hands

My husband was too sick by this time to know anything except that he was very sick, but I was confident that I knew what he would want.

We were taking a risk to do this, rather than go to a local hospital.

Each person and their loved ones must make

what they believe is the right decision,

and be prepared to accept the consequences, whatever happens.

I rode in the ambulance with him, seated at his head, steadfast in the conviction that he needed to return to these specific doctors. I did not know what was at the root of this. However, especially if he had succumbed to an infection, I wanted him in the hands of his surgeon, who has a stellar track record at conquering post operative infections.

Mercifully, his vital signs remained stable, and what seemed an endless ride at last brought us to the Door of Hope pictured above. He remembers almost nothing of any of this - the ambulance ride, the experience in the Emergency Department itself, and some of the time in Intensive Care. I am so glad. It is enough that I must remember it.

In the Doctors' Hands

I stayed in the Emergency area to answer questions and sign any permissions needed to treat him. I can never express my gratitude to the Emergency doctor, in my memory a lovely woman with dark hair, and the two nurses that worked on him. It was not long before the Pulmonary specialist who had cared for him appeared. I am sure he was shocked to see the change in a man who had looked so well when discharged. I know I was crying as I told him that my husband was just "a mess"! Later his surgeon arrived, and no doubt others.

It was up to these doctors now, to do what they could. And they did!

Acute Renal Failure

The Mayo Clinic website states that this can happen rapidly, can be deadly, and requires intense treatment. I certainly saw the reality of those words!

In that cubicle in the Emergency Department, and then in Intensive Care and through out his hospitalization, the treatment was indeed intense.

I have not counted the number of doctors who cared for him. There were many.

Grateful

Today we are grateful for many things, especially the expert and compassionate treatment and care. His doctors and nurses gave him the best possible treatment and recovery. We are home together again, no dialysis was ever needed!

Surgery Far From Home

Out of four heart surgeries, only one was done nearby our home. From past experience, I knew that it might be necessary to return to his doctors and hospital if there were complications. In 2006, we had signed out of our local hospital, who wanted to admit him, and I drove him back to the hospital where he had surgery myself. He was stable enough and well enough to ride in the car that time.

However, this time, he was too ill, too quickly, for me to drive him there. What I discovered is that, even if paid privately, cash up front, many ambulance services will not transport someone from their home to the hospital they request.

If it should be necessary again, I will always have the phone number of that inter facility transport service in my area at hand and be prepared to call them.

The 911 service responds to everyone, and takes them to the nearest hospital equipped to help them. For example, in the case of a stroke, to the nearest stroke designated hospital. Generally this makes sense.However, in a case such as this, wishing to return after a major heart surgery to his doctors was a challenge. It was necessary to pay the full ambulance fee up front in order to make it happen. I understand that may be beyond what many people could do. In that case, the next best thing is to get to the local hospital via 911.Let Others Help You
Normally, when we are well, we are used to being independent. Going through this crisis, there were so many who were so thoughtful and helped so much. We can never adequately thank those who were there in our hour of need.

I think now of my dear friend and her husband, who drove my car to the hospital that day while I rode in the ambulance, and stayed with me in those first terrible hours at the hospital. Others came later to be with me. A very dear friend of the heart, on the other side of the world, spoke to me by phone at midnight that night. A very brave young woman came daily to support and cheer us. There were so many messages of love and encouragement.

For all those who sent thoughts and prayers on our behalf, we are forever grateful.

Plan for the Worst, Hope for the Best!

Thinking about it now, I could have identified the ambulance company ahead of time. I just did not realize many would not choose to be hired privately. And so, in the future for us, when our doctors are not nearby, I will plan for the worst case, and hope for the best!

And that worst case would include knowing ahead of time how to request an ambulance to take us back to the doctors and hospital we need.

Arlys Velebir

Chairman & President of BAF

Disclaimer

Arlys Velebir is the President and Chairman of the Bicuspid Aortic Foundation. The statements and opinions presented here are entirely her own personal thoughts, and do not in any way represent the position of the Bicuspid Aortic Foundation, its Board of Directors, Scientific Advisory Board, or volunteers.